Intestinal Vascular Malformations Research Articles

A100 HEREDITARY HEMORRHAGIC TELANGIECTASIA: MORE THAN JUST ANOTHER RECTAL BLEED

Abstract Background Hereditary hemorrhagic telangiectasia (HHT), previously known as Osler-Weber-Rendu syndrome, is an autosomal dominant disorder characterized by widespread telangiectasia and visceral arteriovenous (AV) malformations. Diagnosis of HHT is commonly guided by The Curaçao Diagnostic Criteria. Molecular genetics can help confirm the clinical diagnosis. Genes involved include ENG, ACVRL1, SMAD4, and GDF2, which affect the bone morphogenic protein signaling pathway important in maintaining vascular endothelial integrity. Aims This report aims to review the case of a young male from a rural community in Northern Alberta with a possible new diagnosis of HHT, presenting with rectal bleeding in the setting of family history of telangiectasias. Methods Retrospective review of one patient. Results We report the case of a 33-year-old male who presented with a 1-day history of rectal bleeding (bright red turning to black) with hemoglobin at 116g/L. Past medical history was significant for occasional epistaxis since age 8, previous gastrointestinal bleed and laparotomy with pathology showing small intestinal vascular malformation at the age of 15, and small bowel obstruction secondary to adhesions requiring resection at the age of 26. There was no history of iron deficiency anemia. Family history was positive for a mother who had Von Willebrand disease and partial colectomy at the age of 28 due to bowel telangiectasias. His mother had no formal HHT diagnosis, but it was unclear if molecular genetics had been performed. Inpatient colonoscopy showed no culprit lesions but free-floating blood clots throughout the colon (Image). EGD and push enteroscopy were normal. CT enterography was then performed showing AV fistula in the distal ileum and venous malformations in the jejunum. Video capsule endoscopy followed revealed a few non-bleeding telangiectasia and petechiae. The patient was assessed to have possible HHT by The Curaçao Diagnostic Criteria. He was supplemented with IV iron and started on short-acting octreotide. On discharge, he received funding approval for long-acting octreotide and was referred to the Edmonton HHT clinic for follow up and further screening. Conclusions HHT remains underdiagnosed in both children and adults. Maintaining a strong suspicion and prompt diagnosis via the Curaçao Criteria is crucial for appropriate screening of the multisystem complications which stem from the AV malformations. This multidisciplinary approach can aid in reducing morbidity and mortality. Funding Agencies None

Convolutional neural network-based segmentation network applied to image recognition of angiodysplasias lesion under capsule endoscopy

Small intestinal vascular malformations (angiodysplasias) are common causes of small intestinal bleeding. While capsule endoscopy has become the primary diagnostic method for angiodysplasia, manual reading of the entire gastrointestinal tract is time-consuming and requires a heavy workload, which affects the accuracy of diagnosis. To evaluate whether artificial intelligence can assist the diagnosis and increase the detection rate of angiodysplasias in the small intestine, achieve automatic disease detection, and shorten the capsule endoscopy (CE) reading time. A convolutional neural network semantic segmentation model with a feature fusion method, which automatically recognizes the category of vascular dysplasia under CE and draws the lesion contour, thus improving the efficiency and accuracy of identifying small intestinal vascular malformation lesions, was proposed. Resnet-50 was used as the skeleton network to design the fusion mechanism, fuse the shallow and depth features, and classify the images at the pixel level to achieve the segmentation and recognition of vascular dysplasia. The training set and test set were constructed and compared with PSPNet, Deeplab3+, and UperNet. The test set constructed in the study achieved satisfactory results, where pixel accuracy was 99%, mean intersection over union was 0.69, negative predictive value was 98.74%, and positive predictive value was 94.27%. The model parameter was 46.38 M, the float calculation was 467.2 G, and the time length to segment and recognize a picture was 0.6 s. Constructing a segmentation network based on deep learning to segment and recognize angiodysplasias lesions is an effective and feasible method for diagnosing angiodysplasias lesions.

Obscure Gastrointestinal Bleeding Caused by a Small Intestinal Lymphatic-venous Malformation: A Case Report with a Literature Review

A 44-year-old woman presented with severe anemia. We strongly suspected gastrointestinal bleeding; however, esophagogastroduodenoscopy, colonoscopy, and computed tomography showed no bleeding sources. Video capsule endoscopy revealed an actively bleeding submucosal lesion within the jejunum. Double-balloon enteroscopy revealed a 20-mm continuously bleeding submucosal lesion in the distal jejunum. We suspected small intestinal vascular malformation and performed surgical resection. The resected specimen pathologically comprised dilated, thin-walled lymphatic channels and blood vessels involving the small intestinal submucosa. Therefore, the patient was diagnosed with small intestinal lymphatic-venous malformation. Postoperatively, the patient recovered well, and recurrence was not observed.

Application of capsule endoscopy in patients with chronic and recurrent abdominal pain: Abbreviated running title: capsule endoscopy in abdominal pain.

The incidence of chronic and recurrent abdominal pain increases every year, while the diagnosis is still unsatisfactory even after a number of check-ups. This study aimed to evaluate the diagnosis value of capsule endoscopy in patients suffering from chronic and recurrent abdominal pain. A retrospective case study was performed in 80 chronic and recurrent abdominal pain patients at Xiangyang Central Hospital from January 2013 to November 2017. Meanwhile, diagnoses by capsule endoscopy were collected for analysis. Abnormal findings were found in 54 of 80 (67.5%) patients. The findings in chronic and recurrent abdominal pain patients include small intestinal erosion and congestion, small intestinal ulcers, small intestinal parasites, small intestinal vascular malformations, small intestinal polyps, small intestinal diverticulum, and small intestinal lymphangiectasia. There were no immediate significant side effects without being reported up to 1 month after ingestion of the capsule. The capsule was evacuated by all patients. Capsule endoscopy has a great value in the diagnosis of chronic and recurrent abdominal pain with satisfactory safety and less pain for patients. Inflammatory lesions and ulcers in the small intestine account for the majority of positive findings in these patients.

Dysphagia in an Adolescent with Iron Deficiency Anemia.

Dysphagia in an Adolescent with Iron Deficiency Anemia.

How to do a modified vascular malformation suture for multiple intestinal lesions in blue rubber bleb nevus syndrome without bowel resection.

Intestinal resection surgery for multiple intestinal vascular malformations in the blue rubber bleb nevus syndrome is traumatic and time-consuming. This study and Video S1 introduce a novel vascular malformation suture method to manage this problem without bowel resection.

Re-bleeding events in patients with small intestinal vascular malformation: Risk factors and effect of treatment with thalidomide

Re-bleeding events in patients with small intestinal vascular malformation: Risk factors and effect of treatment with thalidomide

Reason analysis of reoperation after failed laparoscopic surgery for acute abdomen

To analyze the reasons for reoperation after failed laparoscopic surgery for acute abdomen. A retrospective study was performed to review the clinical data of 17 patients who required reoperation after laparoscopic surgery from May 2006 to March 2011. There were 11 males and 6 females with 16-68 years old. The reasons for reoperation were as follows: 13 with abdominal abscess among whom 4 complicated with internal fistula, 1 with residual toothpick, 1 with ileocecal lymphadenoma complicated with bowel obstruction, 1 with obturator hernia, 1 with intestinal vascular malformation. Twelve patients underwent laparoscopic reoperation after conservative treatments were invalid, among whom 5 completed laparoscopic operation successfully and 7 converted to laparotomy. The other 5 patients underwent emergency abdominal operation. All the patients were cured and discharged from hospital 7-21 days postoperatively. The laparoscopic technique has unique advantages, but must strictly adhere to the indications of Laparoscopic exploration and strictly follow the surgical operation procedures. In the special condition,and in diagnosis and surgery difficulty,laparoscopic surgery should be timely and decisive conversion to open surgery in the diagnosis and treatment of surgical acute abdomen.

Intestinal microvascular malformations and congenital asplenia in an adolescent possibly expanding the phenotype of Ivemark syndrome

Intestinal vascular malformations in children and adolescents are rare but must be considered in the differential diagnosis of gastrointestinal bleeding and chronic anemia. We report a 16-year-old girl who developed chronic iron-deficiency anemia due to recurrent bleeding from multiple angiodysplastic lesions of the stomach, duodenum, and jejunum. The cause of blood loss remained unclear for several years while the girl received numerous blood transfusions. Diagnosis was finally established by capsule endoscopy and double-balloon enteroscopy. Treatment was effectively carried out by argon plasma coagulation. Many systemic vascular malformation syndromes are associated with gastrointestinal lesions. However, as no extraintestinal vascular lesions were present in our patient, diagnosis of a known vascular malformation syndrome seemed unlikely. In addition to the microvascular intestinal malformations, we found a familial congenital asplenia without apparent infectious complications. Thus, the reported case possibly constitutes a so far unpublished variant of the Ivemark syndrome without macrovascular malformations but instead with microvascular malformations. We therefore envison that in times of refined diagnostic techniques the phenotype of the Ivemark syndrome might be expanded by including microvascular malformations.

Small intestinal vascular malformation bleeding: diagnosis by double-balloon enteroscopy combined with abdominal contrast-enhanced CT examination

AimThe purpose of this study was to explore the value of double-balloon enteroscopy combined with abdominal vascular-enhanced CT examination for the diagnosis of intestinal vascular malformation bleeding, to explore a simple and effective method for the diagnosis of small intestinal vascular malformation bleeding.MethodsTen patients with intestinal bleeding were first examined with double-balloon enteroscopy. If active bleeding considered as vascular malformation was observed, the patient underwent abdominal vascular-enhanced CT examination. If no active bleeding was observed with double-balloon enteroscopy, the patient also underwent abdominal vascular-enhanced CT examination. When intestinal vascular malformation bleeding was diagnosed with double-balloon enteroscopy and/or abdominal vascular-enhanced CT examination, the patient underwent surgical operation and vascular malformation was confirmed with pathologic diagnosis.ResultsIn ten patients who underwent double-balloon enteroscopy examination, active intestinal bleeding was observed in seven patients and no active bleeding was observed in three patients. All ten patients underwent abdominal vascular-enhanced CT examination and vascular malformation was detected in all the patients with confirmation by pathologic diagnosis.ConclusionDouble-balloon enteroscopy combined with abdominal vascular enhanced CT examination is a simple and effective method for the diagnosis of intestinal vascular malformation bleeding.

Assessment of intestinal vascular malformations in patients with hereditary hemorrhagic teleangiectasia and anemia

Hereditary hemorrhagic teleangiectasia (HHT) is an autosomal dominant disorder with mucocutaneous teleangiectasia and visceral arteriovenous malformations. Mutations of endoglin and Activin A receptor like kinase-1 have different phenotypes, HHT1 and HHT2, respectively. The gastrointestinal tract is frequently affected, but limited information is available on the relationship with genotype. To determine whether different genotypes have different phenotypes with respect to intestinal teleangiectasia. HHT patients, referred for anemia, underwent videocapsule endoscopy. Chart review was performed for information on genotype and HHT manifestations. Twenty-five patients were analyzed (men/women 13/9, mean age 49+/-15 years.), 14 HHT1, eight HHT2 and three without known mutation. Epistaxis occurred in 96% of patients. Gastroduodenoscopy revealed teleangiectasia in 7/12 (58%) HHT1 and 3/8 (38%) HHT2 patients. Videocapsule endoscopy found teleangiectasia in all HHT1 and 5/8 (63%) HHT2 patients. In 9/14 HHT1 patients, teleangiectasia were large. Teleangiectasia in the colon was restricted to 6/11 (55%) HHT1 patients. Hepatic arteriovenous malformations were present in 1/7 HHT1 and 5/6 HHT2 patients. Large teleangiectasia in small intestine and colon appear to occur predominantly in HHT1. Hepatic arteriovenous malformations are mainly found in HHT2. In HHT patients with unexplained anemia, videocapsule endoscopy should be considered to determine the size and extent of teleangiectasia and exclude other abnormalities.

New Endoscopic Techniques for Obscure Gastrointestinal Bleeding

The case of a postmenopausal woman with a congenital aortic stenosis is presented. She presented with severe iron deficiency anemia. After negative extensive gastrointestinal analysis, she was treated with octreotide for six months. After cessation of octreotide, anemia rapidly recurred. A second capsule endoscopy and a double balloon enteroscopy were performed, and an intestinal vascular malformation was found. After surgical segment resection, the patient had stable, normal levels of hemoglobin and no complaints after 14 months of follow-up.

Assessment of Intestinal Vascular Malformations in Patients with Hereditary Hemorrhagic Teleangiectasia and Anaemia

Introduction: Hereditary hemorrhagic teleangiectasia (HHT), or Rendu Osler Weber's disease, is an autosomal dominant disorder with mucocutaneous teleangiectasia and visceral arteriovenous malformations. Mutations of endoglin and Activin A receptor like kinase 1 lead to different phenotypes, HHT1 and HHT2 respectively. The gastrointestinal tract is frequently affected which can result in iron deficiency anaemia. There is limited information on the relationship between gastrointestinal/small intestinal teleangiectasia and the genotype. Aim: To determine whether different genotypes HHT have different phenotypes with respect to the presence of small intestinal teleangiectasia as assessed by videocapsule endoscopy. Methods: HHT patients, referred for anaemia, were analysed by videocapsule endoscopy (Given Imaging, Yoqneam, Israel). A chart review was performed to obtain information on the genotype and the presence of HHT manifestations in nose, brain, lungs, liver, upper and lower gastrointestinal tract. Results: Twenty five patients were analysed (M/F 13/9, mean age 49 +/− 15 yrs., range 18-72 yrs.). Fourteen patients had HHT1 and 8 patients had HHT2. In three patients no known mutation was found. The mean haemoglobin level was 7.2 +/− 1.7 mmol/l. Epistaxis occurred in 24/25 (96%) of patients. Gastroduodenoscopy revealed the presence of teleangiectasia in 7/12 (56%) HHT1 patients and in 3/8 (38%) of HHT2 patients. VCE found small intestinal teleangiectasia in all HHT1 patients and in 9/14 patients they were classified as large. In HHT2 patients 5/8 (63%) had small intestinal teleangiectasia, in all but one patient these were small. Vascular abnormalities in the colon occurred in 6/11 (55%) of the HHT1 patients. No abnormalities of the colon were observed in the HHT2 patients. Hepatic arteriovenous malformations were present in 1/7 HHT1 patients and in 5/6 HHT2 patients. Arteriovenous malformations outside the digestive tract were present in the lungs of 9 HHT1 patients and 2 patients with an unknown mutation, and in the cerebrum of 2 HHT1 patients and 1 patient without identified mutation. Conclusion: Teleangiectasia are common in the gastrointestinal tract of HHT patients. The presence of large teleangiectasia in the small intestine and the colon is restricted to HHT1 patients in this study. HAVM predominantly occurs in HHT2 patients. VCE should be performed in HHT patients with anaemia to determine the extent of teleangiectasia.

A case of diffuse intestinal vascular malformation presenting as severe occult gastro-intestinal bleeding

Journal Article A case of diffuse intestinal vascular malformation presenting as severe occult gastro-intestinal bleeding Get access M W H Patterson, M W H Patterson Manchester Royal Infirmary Search for other works by this author on: Oxford Academic Google Scholar J S Whittaker J S Whittaker Manchester Royal Infirmary Search for other works by this author on: Oxford Academic Google Scholar British Journal of Surgery, Volume 53, Issue 11, November 1966, Pages 997–999, https://doi.org/10.1002/bjs.1800531113 Published: 08 December 2005

Bleeding in children caused by gastrointestinal vascular lesions.

Intestinal vascular malformations in infants and children are rare but must be considered in the differential diagnosis of gastrointestinal bleeding. Many vascular malformation syndromes have associated intestinal lesions. Localization of vascular lesions is essential for successful management. A variety of treatment options including laser photoablation, surgical resection, and other nonoperative therapies have been utilized to treat these disorders.

Life-threatening hematochezia from a rectosigmoid vascular malformation in Klippel-Trenaunay syndrome: Long-term palliation using an argon laser

Recurrent severe hemorrhage from intestinal vascular malformations, although extremely rare, may be associated with significant morbidity and mortality. The treatment may require extensive surgical resection, vascular embolization, and repeated blood transfusions. An adolescent boy with the Klippel-Trenaunay syndrome involving the pelvis and left leg presented with recurrent life-threatening hematochezia associated with defecation. Endoscopy documented that the bleeding originated from the submucosal venous angiomata in the region of the hemorrhoidal plexus. An argon laser was used to systematically coagulate the venous angiomata involving the distal 7 cm of the anorectal canal. Postoperative minor rectal bleeding and rectal tenesmus resolved in a few days. The patient has had only one brief episode of hematochezia in more than 4 years of follow-up. The use of the argon laser has provided effective palliation of colorectal vascular malformations with minimal morbidity but more importantly has allowed the preservation of normal anorectal function.

Vascular Disease of the Gastrointestinal Tract: Pathophysiology, Recognition and Management

This text represents a second and expanded edition of the author's Intestinal Ischemia , published in 1977, and in addition to a new title includes special chapters by various experts on intestinal blood flow, testing of intestinal viability, acute intestinal ischemia, pseudomembranous colitis, and neonatal necrotizing enterocolitis. Despite the change in title, this edition remains devoted to discussing the processes and clinical pictures of bowel diseases related to ischemia. It does not cover the problems of intestinal bleeding per se or those associated with intestinal vascular malformations, whether acquired or congenital. For readers seeking information about the physiology of intestinal blood flow, the chapter by Ove Lundgren, University of Goteborg, is especially cogent, and throughout the text the author skillfully promotes the union of basic research with understanding the varying and perplexing clinical diseases. The textual material will appeal to and benefit gastroenterologists, general surgeons, and vascular surgeons who treat lesions

Intestinal Vascular Malformations as Source of Bleeding

Intestinal Vascular Malformations as Source of Bleeding